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BACKGROUND:
Sarcomas of the bladder and prostate comprise a rare disease entity amongst pediatric patients. There is a scarcity of literature describing the epidemiology and natural history of this disease. The purpose of this study is to assess the incidence, clinical characteristics, and survival rates of this rare disease entity.
METHODS:
The United States’ Surveillance, Epidemiology, and End Results (SEER) database was used to identify patients ≤21 years old diagnosed with sarcoma of the bladder or prostate from 1973-2012. Kaplan-Meier method and the log-rank test were performed to assess survival. Multivariate analysis was accomplished using Cox proportional hazard regression. All analyses were performed with SPSS® version 19.0 (SPSS Inc, Chicago, IL, USA).
RESULTS:
There were 168 patients identified, comprised of 94 bladder and 74 prostate tumors. Median age for patients was 3 yrs old (range 0-21yrs). Overall, a majority of patients were aged 0-5 (63.1%), Caucasian (63.1%), and non-Hispanic (83%). The most frequent histology was rhabdomyosarcoma in 91.1%. Bladder tumors occurred more frequently in boys (61.7%) than girls. A statistically significant relationship was seen between histologic subtype and tumor location, with alveolar rhabdomyosarcoma occuring more frequently in the prostate (13.7%), and leiomyosarcoma occuring more frequently in the bladder (10.3%)(p=0.02). Overall 5-year survival for the cohort was 66.3%. Survival was better for bladder tumors (78.9%) than prostate tumors (51.8%)(p<0.001). Overall 5-year survivial for embryonal RMS was 69.4, and leiomyosarcoma 62.2% .An improvement in survival was seen over time, from 46.3% in 1973-1979 to 64.3% in 2000-2012 (p=0.02).
CONCLUSIONS:
Sarcomas of the bladder and prostate are very rare in the pediatric population. Bladder tumors, of all subtypes, have a better prognosis than prostate sarcomas. Our data demonstrates an improvement in survival over time, likely due to advances in multimodal therapies.